Purpose: The thoracic aorta is an important site of atherosclerotic disease
in patients with homozygous familial hypercholesterolemia (HFH). Thoracic
aortic atherosclerosis in patients with HFH was assessed with contrast-enha
nced MR angiograms using exoscopic and endoscopic virtual angioscopy recons
tructions and maximum intensity projections (MIPs).
Method: Contrast-enhanced MR angiograms of the thoracic aorta of 15 patient
s with HFH and 8 normal volunteers were obtained. Perspective surface recon
structions of the MR angiograms including virtual angioscopy views were eva
luated by three radiologists blinded to the diagnosis.
Results: Thoracic wall irregularity was depicted on 8 of 15 (53%) patient s
cans and only 1 of 8 (13%) normal subject scans using surface reconstructio
ns. Wall irregularity scores of patients with HFH were significantly increa
sed compared with controls (2.0 +/- 0.9 vs. 1.0 +/- 0.6; p = 0.008). There
was excellent interobserver agreement (weighted kappa = 0.82 +/- 0.12). Vir
tual endoscopy views added diagnostic confidence compared with exoscopic su
rface renderings alone. MIP reconstructions were unable to depict wall irre
gularity.
Conclusion: MR angiography with virtual angioscopy of the thoracic aorta de
picts nonstenotic wall irregularity of thoracic aortic atherosclerosis in p
atients with HFH. This may be important For assessing disease progression a
nd response to treatment and may be generalizable to routine (non-HFH) athe
rosclerosis.